Merrf Syndrome and Refractory Status Epilepticus

MERRF syndrome (or Myoclonic Epilepsy with Ragged Red Fibers) is a mitochondrial disease maternally-inherited. In this progressive disorder, status epilepticus is common and highly resistant to treatment including generalized anesthesia. We report two cases with Merrf syndrome (sister and brother), presented with status epilepticus refractory even to Thiopental, Propofol and Midazolam. In the same family is and another girl, diagnosed with Merrf syndrome. Given that it wasnt possible to stop the refractory status in these cases, we suggest Ketamine as an alternative to general anesthesia as for blocking seizure activity, as for the potential neuroprotective effect.